Large Mass in Left Atrium by Transesophageal Echocardiography: An Unusual Case of Cardiac Angiosarcoma Mimicking a Cystic Mass

Primary cardiac tumors are extremely rare. Among them, malignant tumors constitute approximately 15% of primary cardiac tumors. Angiosarcomas are the most frequent. They often appear as mural masses in the right atrium and completely replace the atrial wall and fill the entire cardiac chamber. We report an unusual case of angiosarcoma in a 75‐year‐old woman with a cystic appearance, located in the left atrium and causing critical mitral stenosis. (Echocardiography 2010;27:E137‐E138)     

Cyanosis when head turned to left in an infant with primary ciliary dyskinesia

A 19‐month‐old boy with primary ciliary dyskinesia (PCD), mirror‐image dextrocardia, situs inversus (SI) totalis suffered from persistent hypoxia. Cyanosis became clinically evident when the child turned the head to the left and resolved after turning the head to the right. Echocardiography demonstrated two superior caval veins; the left sided superior vena cava (SVC) entering the left sided right atrium (RA) and the right sided into the right sided left atrium (LA). Surgical redirection of the right sided caval vein into RA was performed. Pediatr Pulmonol. 2013; 48:88–90. © 2012 Wiley Periodicals, Inc.     

Carcinoid heart disease and the utility of 3D trans-thoracic and trans-esophageal echocardiography: Two clinical cases

Carcinoid tumors are rare neuroendocrine malignancies. We present two cases of metastatic carcinoid tumors, complicated by carcinoid syndrome and by cardiac valve involvement. Carcinoid syndrome is characterized by secretory diarrhea, episodic flushing, and bronchospasm. Cardiac involvement occurs in up to 50% of patients with metastatic carcinoid tumors which commonly causes abnormalities of the right sided valves. Echocardiography is the best available and non invasive technique for diagnosis. Characteristic features of carcinoid heart disease are thickened, shortened, retracted, and fixed or partially fixed valve leaflets. Three-dimensional (3D) echocardiography provided an en face view of pulmonary and tricuspid valve, not obtainable by two-dimensional echocardiography, and improved delineation of the relationship between these structures and cardiac chambers.     

Transesophageal versus transthoracic echocardiography in the evaluation of right atrial tumors

The purpose of this study was to define the contribution of transesophageal echocardiography to the diagnosis of right atrial tumors in a large series of patients with this rare finding. Transesophageal echocardiography (TEE) has been found to be valuable in evaluating patients with intracardiac masses and has been shown ton be superior to transthoracic echocardiography (TTE) in evaluating left heart masses. Between 1989 and 1993, 23 patients with either known tumors elsewhere or right atrial masses that were detected on TTE were evaluated with TEE. TEE studies were performed in the noninvasive cardiology laboratory. All patients were studied with 5 MHz biplane or omniplane transducers. The right atrium was evaluated for the presence, characteristics, extent, and attachment of any masses and for extension of these masses into the great vessels or other cardiac chambers. No complications occurred. Six patients had primary right atrium tumors. In 10 patients the tumors reached the right atrium via the inferior vena cava. Seven patients had malignant secondary tumors. TEE demonstrated three tumors not detected by TTE. Furthermore TEE provided 16 additional findings not seen on TTE. In conclusion, TEE is superior to TTE in the evaluation of right atrial tumors. TEE should be considered in patients with right atrial tumors even when these tumors have been demonstrated with TTE.     

Intravenous Leiomyomatosis with Intracardiac Extension: Echocardiographic Study and Literature Review

Uterine leiomyomatosis is a common disease in women; however, intravenous leiomyomatosis with intracaval and intracardiac tumor extension is rare. We sought to analyze the clinical and echocardiographic features of intracardiac leiomyomatosis.From January 2003 through July 2012, 7 women (age range, 24–59 yr) underwent surgical resection of histopathologically diagnosed intracardiac leiomyomas at our hospital. Most of the patients had histories of hysterectomy or uterine leiomyoma. We retrospectively analyzed their preoperative echocardiograms. We found that the tumors had no stalks, did not adhere to the wall of the right side of the heart, were highly mobile, and moved back and forth in the right atrium near the tricuspid orifice. All tumors originated from the inferior vena cava and had borders well demarcated from that structure''s wall. Most of the masses extended into the inferior vena cava and right atrium through the right internal and common iliac veins. Computed tomograms revealed pelvic tumors and contiguous filling defects in 6 patients.When echocardiograms reveal a right-sided cardiac mass that originates from the inferior vena cava, particularly in women who have a history of hysterectomy or uterine leiomyoma, intracardiac leiomyomatosis should be suspected. If the mass has no stalk and freely moves within the inferior vena cava and right-sided cardiac chambers without attachment to the endothelial surface or endocardium, intracardiac leiomyomatosis should be diagnosed. We discuss our findings and briefly review the relevant medical literature.     

Right-left shunt caused by sarcoma of the right atrium

A 70 year old woman was admitted for right ventricular failure and cyanosis of recent onset. Echocardiography showed a very large, homogenous, immobile, smooth-contoured mass filling the right atrium. Right atrial pressures were raised but the other intracardiac pressures were normal at catheterisation. Right heart angiography confirmed the voluminous right atrial mass and dilatation of the hepatic veins and showed early opacification of the left heart chambers. A right-to-left shunt was confirmed by oximetry which showed significant desaturation of the blood in the left atrium and ventricle (saturation 78% in the left ventricle). The tumour was also documented by a thoracic CT scan. At surgery, a very large, malignant right atrial tumour was resected which histological examination showed to be an angiosarcoma. The interatrial septum seemed to be intact: there was no true atrial septal defect but a persistent foramen ovale was found. After resection of the tumour the right atrium was reconstructed. The initial postoperative period was uncomplicated but the patient died nine months later of metastatic disease particularly affecting the liver and brain. The clinical presentation of malignant cardiac tumour is very variable but a right-to-left interatrial shunt through a patent foramen ovale has only been reported previously in 2 cases of primary malignant tumours (a rhabdomyosarcoma and an angiosarcoma) and in 1 case of a secondary cardiac metastasis.     

心脏肿瘤外科治疗45例临床分析及右胸切口手术经验总结

目的：探讨心脏肿瘤临床特点, 总结外科治疗经验。方法：回顾分析2008年1月至2017年6月我科45例心脏肿瘤手术患者（3例为复发患者）的临床资料,比较右胸前外侧切口与胸骨正中切口手术的疗效。结果：本组病例中男性19例（42.2%）,年龄52±18岁,术前9例（20%）合并心功能不全。左房占位33例,右房占位9例,左室、右室和多发占位各1例。胸骨正中切口34例,右胸前外侧切口微创手术11例。全组无围术期死亡,右侧微创切口与正中切口组相比,手术时间、术后恢复和术后并发症发生率均无显著差异。全组良性肿瘤42例（93.3%）,其中粘液瘤33例;恶性肿瘤3例（6.7%）。随访无死亡、无复发。结论：心脏肿瘤良性疾病多见,手术效果优良,完整切除是预防复发的重要因素。经右胸微创切口切除心脏肿瘤技术可行,疗效确切,可作为常规胸骨正中切口手术方法的补充。     

Congenitally corrected transposition of the great arteries with anomalous inferior vena cava drainage: multimodality imaging

Atrioventricular discordance with ventricular-arterial discordance is a rare cardiac anomaly known as congenitally corrected transposition of the great arteries (CCTGA). This malformation has a prevalence of 0.4–0.6% of all congenital heart disease cases. Complete heart block develops in up to 30% of patients with CCTGA. We present the case of a 62-year-old woman diagnosed with CCTGA who, on echocardiography, had anomalous venous drainage where the inferior vena cava (IVC) bypassed the right atrium and drained into the azygos system. Complementary images with magnetic resonance imaging demonstrated the unique anatomical relationship between the IVC, azygos venous system, and the superior vena cava. (Echocardiography 2012;29:E16-E19)     

Intravenous leiomyomatosis involving the right heart: Comprehensive evaluation using echocardiography combined with abdominal ultrasonography

Intravenous leiomyomatosis (IVL) is a rare condition characterized by a tumor that originates within a pelvic vein wall or the uterine smooth muscle, with extension into the right heart (referred to as intracardiac leiomyomatosis) in approximately 10% of all IVL cases. Usually, computed tomography (CT) or magnetic resonance imaging (MRI) is performed for diagnostic imaging of IVL. Notably, this neoplasm presents with characteristic ultrasonographic findings. In this report, we present the case of a 49-year-old woman with IVL, which extended into the right heart. Echocardiography combined with abdominal ultrasonography was useful to outline the course of the tumor from the right heart to the uterus. Our findings suggest that in addition to CT or MRI, ultrasonography shows high diagnostic value in cases of IVL, and ultrasonography combined with CT or MRI can further improve the preoperative diagnosis rate of IVL.     

Intravenous leiomyomatosis: A rare cause of intracardiac mass

Intravenous leiomyomatosis is an unusual clinical condition characterized by histologically benign smooth muscle lesions extending from the uterus into pelvic and systemic veins and, more rarely, into the right cardiac chambers. We report the case of a 45-year-old woman who presented with a three-week history of dyspnea on exertion, shortness of breath and fatigue. Echocardiography showed a large mobile mass in the right atrium prolapsing into the right ventricle and extending to the inferior vena cava. A computed tomography scan revealed a large mass extending from the right atrium to the inferior vena cava and through the systemic veins as far as the popliteal veins. A presumptive diagnosis of large thrombus was made; the correct diagnosis of intravenous leiomyomatosis with intracardiac involvement was obtained only after surgical resection and histologic examination.     

Atrial septal aneurysm: a report of three cases

Three cases of isolated atrial septal aneurysm are reported. Case 1: A 66-year-old man visited our hospital for detailed examination and evaluation of premature ventricular contractions and left ventricular hypertrophy previously detected. Echocardiography revealed an atrial septal aneurysm protruding into the left atrium in the early systolic phase and into the right atrium during the mid-systolic to diastolic phase. Subxiphoid M-mode echocardiography showed triphasic movement of the wall of the atrial septal aneurysm toward the left atrium during the end-diastolic to systolic phase, which was enhanced during inspiration. No abnormalities were found by Doppler echocardiography. Cardiac catheterization revealed normal intracardiac pressures and oxygen saturations, but a right arteriogram revealed a defect in the right atrium. Case 2: A five-month-old male infant was referred to our hospital for detailed examination and evaluation of a heart murmur. Echocardiography revealed an atrial septal aneurysm in which a part of the interatrial septum protruded into the right atrium. Case 3: A one-day-old female infant was admitted to our hospital for detailed cardiac examination. Echocardiography revealed that the entire interatrial septum protruded into the right atrium. In Cases 2 and 3 the morphology of the aneurysms did not show cyclic or respiratory change. These two cases had no other abnormalities on echocardiography or Doppler echocardiography. The mechanism of the wall movement in Case 1 seemed to be attributed to differences in pressures between the right and left atria and in properties of the aneurysmal walls. Enhancement of the protrusion of the aneurysmal wall toward the left atrium during inspiration was considered due to inspiratory increase of right atrial pressure.     

Do Pre‐Hemodialysis Estimates of Extracellular Volume Excess Using Bioimpedance and N‐Terminal Brain Natriuretic Peptide Correlate With Cardiac Chamber Size Measured by Magnetic Resonance Imaging?

Bioimpedance can be used to measure extracellular water (ECW) and total body water in hemodialysis (HD) patients and estimate ECW excess. However, ECW excess potentially includes both an increase in the plasma volume and also the extravascular volume. Overestimating the amount of fluid to be removed during HD risks intra‐dialytic hypotension. We wished to determine the association between estimates of ECW excess comparing several different equations using bioimpedance, brain N‐terminal pro‐brain natriuretic peptide (NT‐proBNP) with cardiac chamber volumes and function as determined by cardiac magnetic resonance imaging pre‐HD measurements of ECW and total body water were made using multifrequency bioimpedance and cardiac chamber sizes and function were determined by magnetic resonance imaging. Thirty patients, 20 males (66.7%), mean age 64.4 ± 15.3 years were studied. ECW and ECW/height were positively associated with indexed right ventricular end‐systolic (RVESVi) and end‐diastolic volume (RVEDVi) (RVESi r = 0.46, r = 0.43; RVEDi r = 0.50, r = 0.44, all P < 0.05), but not with left sided cardiac volumes. Whereas NT‐proBNP was associated with indexed left atrial and ventricular size (r = 0.47, r = 0.58, P < 0.05), but not right sided cardiac volumes. Pre‐HD NT‐proBNP was associated with left sided cardiac chamber sizes, but not with right sided chamber sizes, whereas ECW/height was associated with right sided cardiac chamber sizes. As right‐sided cardiac chamber size is more responsive to and reflective of changes in intravascular volume than the left atrium and ventricle, then bioimpedance measured ECW is potentially more reliable in estimating plasma volume expansion.     

Clinical presentation and non-invasive diagnosis of right heart masses.

Right-sided cardiac masses do not have a uniform clinical presentation. Successful diagnosis is dependent on a high index of suspicion and appropriate echocardiographic examination. Five cases of right sided intracardiac masses have been detected during the period that echocardiography has been routinely available to us--two of right atrial myxoma, one of right atrial thrombus, and two of right ventricular tumour. M-mode echocardiography identified four of the five cases. Two-dimensional echocardiography was necessary to establish the non-invasive diagnosis in the fifth case. Echocardiography should be used in any patient in whom a right sided mass is suspected, or in those patients presenting with signs of tricuspid regurgitation, tricuspid stenosis, pulmonary stenosis, cyanotic heart disease, progressive right heart failure, constrictive pericarditis, or pulmonary emboli without obvious source.     

Primary Cardiac Angiosarcoma with Spontaneous Ruptures of the Right Atrium and Right Coronary Artery

Primary tumors of the heart are rarely seen. Cardiac angiosarcomas are malignant tumors that almost always have a poor prognosis. Atrium rupture and coronary artery fistula are very rare complications of primary cardiac angiosarcoma. We describe a 57‐year‐old man suffering from primary cardiac angiosarcoma with spontaneous ruptures of the right atrium and right coronary artery (RCA). Theoretically, either of these ruptures invariably results in pericardial effusion and tamponade that is rare but potentially life threatening. In this instance, however, the patient might have developed fibrous adhesions resulted from previous bloody pericardial effusion. A massive pericardial effusion was localized, which consequently prevented cardiac tamponade and hemodynamic collapse. Echocardiography revealed the tumor progression leading to detectable infiltration of solid mass into the right atrial (RA) wall, which is close to RCA. And color Doppler displayed the flow into the pericardial cavity through a disrupted RA wall and perforated RCA. Echocardiography remains the primary method of choice for evaluation of cardiac masses.     

Differences between right- and left-sided colon cancer in patient characteristics, cancer morphology and histology

Background and Aim: Recently, the clinical and biological differences between right‐ and left‐sided colon cancers have been widely debated. However, close analyses of these clinical differences, based on large‐scale studies, have been scarcely reported. Methods: A total of 3552 consecutive Japanese colorectal cancer cases were examined and the clinical differences between right‐ and left‐sided colon cancer cases were investigated. Results: The proportion of right‐sided colon cancer was relatively high in patients aged less than 40 years (33%) and more than 80 years (43%). The proportion of right‐sided colon cancer in patients aged 40–59 years was relatively low (male 22% and female 29%). In male patients the proportion increased in the 70‐79 years age group (30%), while in female patients the proportion increased in the 60‐69 years age group (39%). Right‐sided colon cancer was more likely to be detected at an advanced stage (T1 stage; left 22%, right 15%) (P < 0.01) with severe symptoms. Polypoid‐type early cancer was dominant in the left colon (left 59%; right 40%) (P < 0.01), while the proportion of flat‐type early cancer in the right colon was significantly higher than that in the left colon (left 25%; right 44%) (P < 0.01). Conclusions: Specific age distribution of right‐sided colon cancer was observed and the difference between male and female patients was highlighted. Other clinical features also differed between right‐ and left‐sided colon cancer, suggesting that different mechanisms may be at work during right and left colon carcinogenesis.     

Surgery of hepatoma with intracavitary cardiac extension

Summary A case of primary liver carcinoma with intracavitary cardiac extension is presented. A 36-year-old female was admitted to our surgical clinic with dyspnea and generalized edema. Echocardiography and superior vena cavography demonstrated a large filling defect in the right atrium. After a diagnosis of acute cardiac failure due to an intracardiac tumor, the patient was operated upon immediately. A right atriotomy exposed a large yellow mass within the right atrium, which was not adherent to the atrial wall. The mass was in continuity with similar material in the inferior vena cava and right hepatic vein. With a suspicion of hepatic malignancy, the atrial tumor was removed, and debulking of the mass in the inferior vena cava and right hepatic vein was performed. A postoperative histological examination of the tumor showed hepatocellular carcinoma. Her postoperative course was uneventful, and she was discharged from the hospital.Intracardiac extension of hepatoma is rarely encountered. In this clinical setting, long-term survival cannot be anticipated from any surgery, but palliative clearing of the atrium and inferior vena cava may be of value in preventing cardiac arrest causing sudden death.     

Surgery of the inferior vena cava for tumor-related obstruction

Kidney tumors and pheochromocytomas of the adrenal gland may extend into the inferior vena cava (IVC) or even the right atrium. In these cases, curative surgery frequently requires partial resection of the IVC. From 1992 to 1995, 8 patients (6 kidney tumors, 2 pheochromocytomas of the adrenal gland) underwent tumor surgery with concomitant IVC resection. Four patients had tumor growth into the right atrium. Surgery was carried out during hypothermic circulatory arrest (n=4) using veno-venous bypass from the IVC to the jugular vein (n=2), or by simply clamping the IVC (n=2). In 6 patients the IVC was reconstructed using a PTFE prosthesis (n=2) or patch (n=4), in 2 patients the IVC was closed by a running suture only. All patients were discharged home 10–23 days after surgery (mean 13 days). During the follow-up (mean 23 months), 6 patients were in good clinical condition and without evidence of tumor recurrence up to 44 months after surgery and 2 patients died of metastases (16 and 17 months). Refinements of surgical techniques combined with a multidisciplinary approach involving vascular surgeons, cardiac surgeons, and urologists allow successful treatment of advanced tumor stages with IVC involvement, formerly considered a contraindication for surgery. The aggressive surgical approach is warranted not only for relief of symptoms, and acceptable survival rates can be achieved; even cure of the disease is possible.Presented at the 38th Annual World Congress, International College of Angiology, Köln, Germany, June 1996     

Aneurysmal dilatation of the inferior vena cava

Aim: Inferior vena cava aneurysms (IVCA) are rare, unlike aortic aneurysms. The diagnosis and treatment is challenging. This study defines clinical and echocardiographic findings in a prospective cohort of sixteen patients with fusiform IVCA. Methods and Results: All patients referred to the Mayo Clinic between January 2006 and July 2009 for a clinically indicated echocardiogram (36,128 patients) were screened for a dilated IVC. Sixteen cases of fusiform IVCA were identified. Eleven cases (68.8%) were female. Mean age at presentation was 76 years (range 51–89). Eleven (68.8%) had structural heart disease: with right ventricular (RV) dysfunction in 45.5% (n = 5), moderate or greater tricuspid regurgitation (TR) was seen in 36.4% (n = 4) and RV enlargement was seen in 18.2% (n = 2). The most common clinical indication for echocardiography was dyspnea (25%; n = 4) and heart failure (18.8%; n = 3). The mean IVCA diameter was 4.1 cm (range 3.8–5 cm) and the mean length of the aneurysms was 6.2 cm (range 3.5–8.7 cm), with mean right ventricular systolic pressure of 55 mmHg (range 31–105 mmHg). Five (31.3%) had at least a moderate reduction in right ventricular ejection fraction and five (31.3%) had significant TR. Among these five patients with significant TR, severe TR was present in 80%; (n = 4) and moderate to severe TR was present in 20%; (n = 1). Conclusions: IVC aneurysms are more common in the elderly, and is associated with an increase in right sided heart pressures, significant TR, and RV dysfunction. (Echocardiography 2011;28:833‐842)     

Abnormal drainage of inferior vena cava to left atrium together with a partial abnormal pulmonary venous drainage to right atrium in the presence of atrial septal defect

The direct abnormal drainage of the inferior vena cava (IVC), while rare, is well‐recognized anomaly of systemic venous drainage. It has been reported both in isolation and in association with other cardiac defects. This is a case of an abnormal drainage of IVC into left atrium (LA) together with partial abnormal pulmonary venous drainage to the right atrium (RA) and atrium septal defect (ASD).     

Abnormal wall movements of the right ventricle and both atria in patients with pericardial effusion as indicators of cardiac tamponade

A retrospective study on 126 patients with pericardial effusionshowed cardiac tamponade to be present in 39 patients and absentin 87. We evaluated the distribution of the pericardial effusionas well as the effect of hydrodynamic compression on the differentheart chambers. Small to large volumes of pericardial fluidwere found in both groups of patients. Abnormal wall movementswere only present if pericardial effusion was adjacent. Hydrodynamiccompression signs consisted of abnormal wall movements of bothatria at enddiastole continuing into systole, and of the rightventricle in early- and mid-diastole. Most of the patients withcardiac tamponade showed an abnormal motion pattern of bothright atrium and ventricle (13 patients) or of the right atrialwall alone (12 patients). Abnormal motion of the right ventriclealone was seen in 6 patients, of both atria and right ventriclein 4 and of the left atrium alone in 1 patient. False-negativediagnoses of cardiac tamponade occurred in 3 of the 39 patientsand false-positives in 2 of the 87 (sensitivity 92%, specificity98%). Measurements of the duration of the inversion of bothatria increased the specificity of these abnormal wall movementsto 100%. Echocardiography can help to identify those patientswho are clinically at risk and need pericardial drainage.